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Article

Alpha 1-antitrypsin deficiency and liver disease.

Journal of inherited metabolic disease - 1 Jan 1991

Birrer P, McElvaney N G, Chang-Stroman L M, Crystal R G

Abstract excerpt

Alpha 1-antitrypsin (alpha 1AT) deficiency, one of the most common lethal hereditary disorders among Caucasians, is associated with emphysema in adults, while in children it is associated with liver disease. Produced in the liver and released into the plasma, alpha 1AT serves as the body's major inhibitor of neutrophil elastase, a powerful proteolytic enzyme capable of degrading extracellular structural proteins....

Topics

Humans
Liver Diseases
Phenotype
alpha 1-Antitrypsin Deficiency

Source

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Identifiers

PMID: 1749216
DOI: 10.1007/BF01797921

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 29 Aug 2026. Prepared record publication-1749216-2026-08-29.1. Manifest publication-graph-cohort-2026-08-29.1.

Source receipt: PubMed via Helena Literature Corpus, retrieved 29 Aug 2026.

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